The Dyke-Davidoff-Masson syndrome is characterized by various symptoms related to hemiatrophy of the cerebrum and hypertrophy of the ipsilateral calvarium and paranasal sinuses. Clinical findings include hemiparesis or hemiplegia, seizures and/or mental retardation. The present report discusses the very unusual case of a late-diagnosed Dyke-Davidoff-Masson syndrome in a 75-year-old body donor who had suffered a left-sided stroke associated with the internal carotid artery in the course of tonsillitis at the age of 5.